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Neurofibromatosis type 1 (NF1) is a hereditary, progressive and unpredictable disease, which can involve many organs. Benign and malignant tumors arise due to unrestrained cell division and cell growth. Recognizing the symptoms of these tumors and using the correct diagnostics is of great importance. In this clinical lesson we show the disease course of 3 patients with NF1. In all 3, the disease course was complicated by a symptomatic tumor. Characteristic in these patients is the relatively long interval between the onset of symptoms and the final tumor diagnosis. In this clinical lesson we examine the causes of this in more detail and we emphasize the importance of the specific knowledge within the Dutch national NF1 care network.

The chest x-ray (CXR) was the gold standard in the diagnosis of pneumonia in children. However, CXR has limitations and cannot discriminate in etiology. Current guidelines recommend against routine use of CXR in children with uncomplicated lower respiratory tract infections (LRTI). We used routine care data from a multicentre RCT including 597 children with LRTI symptoms, to evaluate the influence of CXR on antibiotic prescription in the emergency department (ED). CXR remains frequently performed in non-complex children suspected of LRTI in the ED (18%). Children who underwent CXR were more likely to receive antibiotics, even when adjusted for symptoms, hospital and CXR results. Our study highlights the inferior role of CXR in treatment decisions for children with LRTI as CXR, regardless of its results, is independently associated with more antibiotic prescriptions.

Objective: To assess the impact of a clinical decision model for febrile children at risk for serious bacterial infections (SBI) attending the emergency department (ED)., Method: Randomized controlled trial with 439 febrile children, aged 1 month-16 years, attending the pediatric ED of a Dutch university hospital during 2010-2012. Febrile children were randomly assigned to the intervention (clinical decision model; n=219) or the control group (usual care; n=220). The clinical decision model included clinical symptoms, vital signs, and C-reactive protein and provided high/low-risks for "pneumonia" and "other SBI". Nurses were guided by the intervention to initiate additional tests for high-risk children. Primary patient outcome was defined as correct SBI diagnoses. Secondary process outcomes were defined as length of stay; diagnostic tests; antibiotic treatment; hospital admission; revisits and medical costs., Results: The decision model had good discriminative ability for both pneumonia (n=33; AUC 0.83 (95% CI 0.75-0.90)) and other SBI (n=22; AUC 0.81 (95% CI 0.72-0.90)). Compliance to model recommendations was high (86%). No differences in correct SBI determination was observed. Application of the clinical decision model resulted in less full-blood-counts (14% vs. 22%, p-value<0.05) and more urine-dipstick testing (71% vs. 61%, p-value<0.05)., Conclusion: In contrast to our expectations no substantial impact on patient outcome was perceived. The clinical decision model preserved however, good discriminatory ability to detect SBI, achieved good compliance among nurses and resulted in a more standardized diagnostic approach towards febrile children, with less full blood-counts and more rightfully urine-dipstick testing. Trial registration: Trialregister.nl: NTR2381.

Febrile children pose the diagnostic dilemma of distinguishing those with serious infections from the vast majority with self-limiting diseases at an early stage. Alarm symptoms can aid in assessing the probability of serious infections in febrile children. The combined absence of alarm symptoms is useful in ruling out serious infections. CRP and PCT values are important diagnostic markers in febrile children in hospital settings. Children without any alarm symptoms and low inflammatory markers can be treated conservatively, provided there are good instructions on reassessment. Children with amber alarm symptoms or mildly elevated inflammatory markers in whom a serious infection cannot be ruled out require clinical observation or ambulant follow-up; empirical parenteral antibiotic treatment should also be considered and easily accessible. Children with red alarm symptoms or highly elevated inflammatory markers deserve clinical observation. In children < 3 months, empirical parenteral antibiotic treatment is also indicated.